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Genetic and Rare Diseases Information Center (GARD)

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Whipple disease


Other Names for this Disease
  • Intestinal lipodystrophy
  • Intestinal lipophagic granulomatosis
  • Secondary Non-tropical Sprue
  • Tropheryma whippelii infection
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Your Question

My brother contracted Whipple disease last year. He has been in the hospital and extended care facilities since then. His left hand is impaired and he has been receiving physiotherapy to help him walk and function again. How are the neurologic manifestations of Whipple disease treated? What is the prognosis?

Our Answer

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What are the neurologic manifestations of Whipple disease?

Neurologic symptoms occur in some people diagnosed with Whipple disease and can mimic symptoms of almost any other neurologic condition.[1] Fewer than 10-15% of patients with Whipple disease develop clinically significant central nervous system involvement.[2]

Neurologic symptoms of Whipple disease include:[1]

  • vision problems
  • dementia
  • facial numbness
  • headache
  • muscle weakness or twitching
  • difficulty walking
  • memory problems
Last updated: 8/1/2012

How might the neurologic symptoms of Whipple disease be treated?

Treatment of patients with Whipple disease should be based on a multi-team approach, targeting early involvement of gastroenterology, neurology, ophthalmology, cardiology, and rheumatology specialists.[2]

People with Whipple disease need to take long-term antibiotics to cure any infections of the brain and central nervous system. An IV dose of an antibiotic called ceftriaxone is often the first line of treatment. This medication is often followed by another antibiotic (such as trimethoprim-sulfamethoxazole which can cross the blood-brain barrier) taken by mouth for at least a 1 year (sometimes up to 2 years).[1][2] Treatments of shorter duration have been associated with a high rate of relapse. The use of specific diagnostic techniques (i.e. PCR) is important in establishing a diagnosis and in evaluating response and adjusting the antibiotic therapy.[2] If symptoms come back during antibiotic use, the antibiotic treatment may be changed.[1]

Relapsing neurologic Whipple disease is sometimes treated with a combination of antibiotics and weekly injections of interferon gamma (IFNγ)—a substance made by the body that activates the immune system.[1]

More details related to the management of the neurologic manifestations of Whipple disease can be accessed through the Treatment and Medication sections of the Medscape Reference article related to this topic.

Last updated: 8/1/2012

What is the outlook for people with neurologic manifestations of Whipple disease?

After treatment, the likely outcome for most people with Whipple disease is good. Most symptoms disappear in about 1 month. Relapse is common, however, highlighting the need to closely watch for a return of symptoms. Endoscopy with small intestinal biopsy followed by PAS staining and electron microscopy or PCR testing should be repeated 1 year after the start of treatment.[1]

People with neurologic Whipple disease who relapse tend to have much poorer health outcomes, including serious neurologic symptoms and even death; therefore, some researchers argue that all cases of Whipple disease should be considered neurologic.[1]

Prompt diagnosis is imperative, as very effective therapies are easy to employ with typically rapid limitation of central nervous system (CNS) progression and even partial reversal of CNS symptoms. If left untreated, progression to death may come as quickly as 1 month after CNS involvement begins.[3]

Last updated: 8/1/2012

References